先天性完全性房室传导阻滞双胎早产儿1例并文献复习  

Case report of congenital complete heart block in premature twins and literatures review

作　　者：何晓敬 李珍珍 石计朋[1] 曹银利[1] 王卫卫 桑桂梅 郭洪旭[1] 尚云[1] 唐成和[1] He Xiaojing;Li Zhenzhen;Shi Jipeng;Cao Yinli;Wang Weiwei;Sang Guimei;Guo Hongxu;Shang Yun;Tang Chenghe(Department of Pediatrics,the First Affiliated Hospital of Xinxiang Medical University,Weihui 453100,Henan Province,China)

机构地区：[1]新乡医学院第一附属医院儿科,河南卫辉453100

出　　处：《中华实用儿科临床杂志》2020年第14期1098-1101,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：新乡医学院第一附属医院青年培育基金(QN-2019-B11)。

摘　　要：目的探讨双胎先天性完全性房室传导阻滞(CCHB)的发病机制及防治措施。方法回顾分析新乡医学院第一附属医院新生儿科收治的1例CCHB双胎早产儿的临床资料,并复习相关文献。结果1.病例回顾:37岁无症状孕妇,孕23周胎儿超声心动图提示双胎心率下降,房室传导阻滞,母亲完善检查诊断为"未分化结缔组织病",予人免疫球蛋白、地塞米松及羟氯喹等治疗后,孕31周胎儿超声心动图仍提示房室传导阻滞,孕32^+3周因先兆早产行剖宫产,双胎自身抗体筛查均示抗核抗体(ANA)弱阳性,Ro60及Ro52阳性,24 h Holter均提示Ⅲ度房室传导阻滞,宝一对症治疗后出院时体质量增加至2200 g,但仍为CCHB(心室率80~90次/min),宝二住院期间突然出现心率及血压下降,最终心脏骤停,抢救无效死亡。2.文献检索:中文文献2例,英文文献9例,其中9例为抗干燥综合征抗体A(SSA)/Ro和抗干燥综合征抗体B(SSB)/La相关的CCHB,2例为特发性CCHB。结论胎盘转移SSA或SSB是双胎发生CCHB的重要机制,其他因素可能也参与其发病进程。目前的治疗方法仍不尽人意,大多患儿需要起搏治疗,早期诊断和产前管理可改善患儿预后。Objective To investigate the pathogenesis,precaution and treatment of neonatal congenital complete heart block(CCHB)in twins.Methods The clinical data of a case of premature twins with neonatal CCHB from the Department of Neonatology,the First Affiliated Hospital of Xinxiang Medical University were retrospectively analyzed and related literature was reviewed.Results(1)Case review:the 37-year-old gravida had no symptoms.Fetal ultrasound cardiogram(fUCG)at 23 weeks of gestation indicated bradycardia and CCHB.Then,the mother was diagnosed with undifferentiated connective tissue disease.After treatment with human immunoglobulin,dexamethasone and hydroxychloroquine,fUCG at 31 weeks of gestation still suggested CCHB.An emergency cesarean section was performed on the diagnosis of threatened preterm labor.With weakly positive neonatal antinuclear antibody(ANA),and positive Ro60 and Ro52 autoantibodies,twins were diagnosed with CCHB by 24 hour-Holter monitors.One of the twins was discharged with CCHB(ventricular rate of 80-90 times/min)after systemic therapy,but the weight increased to 2200 g.The other one of the twins suffered from the sudden decrease of heart rate and blood pressure and finally died of sudden cardiac arrest.(2)Literature search:two cases in Chinese and 9 cases in English were reviewed.Among them,9 cases were sjogren syndrome type A(SSA)/Ro and sjogren syndrome type B(SSB)/La related CCHB,and 2 cases were idiopathic CCHB.Conclusions The placental transfer of anti-SSA or anti-SSB is an important mechanism of neonatal CCHB in twins,and other factors may also be involved.Current treatments are unsatisfactory.Most patients need pacemaker implantation.Early diagnosis and prenatal management can improve the prognosis.

关 键 词：双胎 早产儿 新生儿狼疮 先天性完全房室传导阻滞 

分 类 号：R722.6[医药卫生—儿科]